title: Severe bacterial infection in patients with heterotaxy syndrome.
abstract: OBJECTIVE
  To determine the incidence of sepsis in patients with heterotaxy syndrome.
  
  
  METHODS
  From our institutional database, we identified patients with heterotaxy syndrome and other complex congenital heart disease (CHD) born between 2001 and 2011. Severe bacterial infection was defined as sepsis with positive culture result or infection with abscess formation.
  
  
  RESULTS
  We enrolled 95 patients with heterotaxy syndrome (88 with right atrial isomerism and 7 with left atrial isomerism) and 142 patients with complex CHD. With 1026 person-years follow-up, the 5-year survival was 52% and 65.7% in heterotaxy and complex CHD groups, respectively (P = .239). Community-acquired severe bacterial infection occurred only in heterotaxy syndrome (13 episodes in 10 patients, 3 of whom had spleen noted at imaging study) with 2- and 5 years cumulative severe bacterial infection rate of 9.6% and 14.5%, respectively. The overall mortality rate of those with community-acquired severe bacterial infection was 31%. Pneumococcus and Citrobacter freundii were the most common pathogens. Nosocomial severe bacterial infection occurred in 33.3% of all patients and 12.5% of all procedures. The rates (0.59 and 0.52/100 hospitalization days in heterotaxy and complex CHD group) and the pathogens of nosocomial severe bacterial infection were similar between heterotaxy and complex CHD groups.
  
  
  CONCLUSIONS
  Patients with heterotaxy syndrome are at high risk for community-acquired severe bacterial infection and also have high mortality rate whether the spleen is present or not. The risk of nosocomial severe bacterial infection seems similar to that of patients with other complex CHD.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Shuenn-Nan
authors.initials: SN
authors.lastname: Chiu
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Pei-Lan
authors.initials: PL
authors.lastname: Shao
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Jou-Kou
authors.initials: JK
authors.lastname: Wang
authors.affiliation: Genomics Research Center, Academia Sinica, Taipei, Taiwan.
authors.forename: Hui-Chi
authors.initials: HC
authors.lastname: Chen
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Ming-Tai
authors.initials: MT
authors.lastname: Lin
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Luan-Yin
authors.initials: LY
authors.lastname: Chang
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Chun-Yi
authors.initials: CY
authors.lastname: Lu
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Ping-Ing
authors.initials: PI
authors.lastname: Lee
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan.
authors.forename: Li-Min
authors.initials: LM
authors.lastname: Huang
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital Medical College, National Taiwan University, Taipei, Taiwan. Electronic address: wumh@ntu.edu.tw.
authors.forename: Mei-Hwan
authors.initials: MH
authors.lastname: Wu
mesh_terms: D001424:Bacterial Infections / Q000453:epidemiology* / Q000209:etiology; D003428:Cross Infection / Q000453:epidemiology* / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D059446:Heterotaxy Syndrome / Q000150:complications*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013624:Taiwan / Q000453:epidemiology
keywords: CHD; CT; Computed tomography; Congenital heart disease; LAI; Left atrial isomerism; RAI; Right atrial isomerism